Onpoaree N, Veeravigrom M, Sanpavat A, Suratannon N, Sintusek P. Unremitting diarrhoea in a girl diagnosed anti-N-methyl-D-aspartate-receptor encephalitis: A case report. World J Clin Cases 2020; 8(20): 4866-4875 [PMID: 33195655 DOI: 10.12998/wjcc.v8.i20.4866]
Corresponding Author of This Article
Palittiya Sintusek, MD, MSc, Assistant Professor, Lecturer, Division of Gastroenterology and Pediatric Gastroenterology and Hepatology Research Unit, Department of Pediatrics, Faculty of Medicine, Chulalongkorn University, 1873, Rama 4 Road, Pathumwan, Bangkok 10330, Thailand. palittiya.s@chula.ac.th
Research Domain of This Article
Gastroenterology & Hepatology
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Norrapat Onpoaree, Faculty of Medicine, Chulalongkorn University, Bangkok 10330, Thailand
Montida Veeravigrom, Division of Neurology, Department of Pediatrics, Faculty of Medicine, Chulalongkorn University, Bangkok 10330, Thailand
Anapat Sanpavat, Division of Pathology, Faculty of Medicine, Chulalongkorn University, Bangkok 10330, Thailand
Narissara Suratannon, Division of Allergy, Department of Pediatrics, Faculty of Medicine, Chulalongkorn University, Bangkok 10330, Thailand
Palittiya Sintusek, Division of Gastroenterology and Pediatric Gastroenterology and Hepatology Research Unit, Department of Pediatrics, Faculty of Medicine, Chulalongkorn University, Bangkok 10330, Thailand
Author contributions: Onpoaree N and Sintusek P reviewed the literature and drafted the manuscript; Veeravigrom M contributed to manuscript draft, interpreted the electroencephalography and made clinical correlation with brain images; Sunpavat A interpreted the histopathological data and provided the histopathology images; Surathannon N interpreted the immunologic study; Sintusek P performed endoscopy, provided the endoscopic images; and all authors approved the final manuscript.
Supported byThe Pediatric Gastroenterology and Hepatology Research Unit, Thailand; Grants for Development of New Faculty Staff, Ratchadaphiseksomphot Endowment Fund, Thailand, No. RA63/012; and Thailand Research Fund Thailand Science Research and Innovation, Thailand, No. MRG6280190.
Informed consent statement: Informed written consent was obtained from the patient's guardian (mother) for publication of this report and any accompanying images.
Conflict-of-interest statement: All authors declare that they have no conflict of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist, and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Palittiya Sintusek, MD, MSc, Assistant Professor, Lecturer, Division of Gastroenterology and Pediatric Gastroenterology and Hepatology Research Unit, Department of Pediatrics, Faculty of Medicine, Chulalongkorn University, 1873, Rama 4 Road, Pathumwan, Bangkok 10330, Thailand. palittiya.s@chula.ac.th
Received: April 11, 2020 Peer-review started: April 11, 2020 First decision: June 13, 2020 Revised: July 27, 2020 Accepted: September 25, 2020 Article in press: September 25, 2020 Published online: October 26, 2020 Processing time: 194 Days and 4 Hours
Abstract
BACKGROUND
Asymptomatic cytomegalovirus (CMV) infection is common in children; in contrast, in children with a weakened immune system, invasive CMV can occur. This is the first case report of a severe manifestation of CMV esophago-enterocolitis in a girl diagnosed with anti-N-methyl-D-aspartate-receptor (anti-NMDAR) encephalitis who received only a moderate dose of corticosteroid therapy.
CASE SUMMARY
A 12-year-old-Thai girl presented with acute behavioural change and headache for 6 d. Electroencephalogram and positivity for NMDAR autoantibodies were compatible with anti-NMDAR encephalitis. Hence, she received pulse methylprednisolone 10 mg/kg per day for 4 d and continued with prednisolone 1.2 mg/kg per day. On day 42 of corticosteroid therapy, she developed unremitting vomiting and diarrhoea. Endoscopy showed multiple ulcers and erythaematous mucosa along the gastrointestinal tract. Tissue CMV viral load and viral-infected cells confirmed CMV esophago-enterocolitis. Therefore, the patient received ganciclovir 5 mg/kg per dose every 12 h for 3 wk and then 5 mg/kg per dose once daily for 3 wk. Unremitting diarrhoea slowly improved from stool output 1-4 L per day to 1-2 L per day after 3 wk of treatment. Pulse methylprednisolone 20 mg/kg for 5 d was initiated and continued with prednisolone 1 mg/kg per day. After this repeated pulse methylprednisolone treatment, surprisingly, diarrhoea subsided. Immunologic work-up was performed to rule out underlying immune deficiency with unremarkable results.
CONCLUSION
Unremitting diarrhoea from CMV esophago-enterocolitis subsided with antiviral and methylprednisolone therapy, implying the immune and NMDAR dysregulation in anti-NMDAR encephalitis.
Core Tip: We report a girl with behavioural change who was finally diagnosed with anti-N-methyl-D-aspartate-receptor (anti-NMDAR) encephalitis. She had unremitting vomiting and diarrhoea after 43 d of corticosteroid therapy and was later diagnosed with cytomegalovirus (CMV) esophago-enterocolitis. Surprisingly, these symptoms subsided after antiviral therapy followed by repeated pulse methylprednisolone targeting the anti-NMDAR encephalitis. CMV esophago-enterocolitis in a patient with anti-NMDAR encephalitis has not been reported before and is very rare in patients who receive only moderate-dose corticosteroids. We highlighted that immune dysregulation in autoimmune encephalitis and the linkage of anti-NMDAR receptors and the enteric nervous system might be the cause of severe gastrointestinal involvement.